Evaluating cochlear implant outcomes in DFNA9 subjects: a comprehensive study on cerebral white matter lesions and vestibular abnormalities.

PURPOSE: This study assessed whether the Fazekas score could account for the variability in cochlear implantation (CI) outcomes among individuals with DFNA9 and evaluated signal loss in the semicircular canals (SCCs) on magnetic resonance imaging (MRI) among individuals with DFNA9. METHOD: This retrospective cross-sectional study included CI recipients with DFNA9. Pre-implantation MRI-scans were reviewed to determine the Fazekas score, localizing and grading cerebral white matter lesions (WML), and identify abnormalities in the SCCs. CI performance was assessed by evaluating phoneme scores one year post-implantation. The function of the SCCs was evaluated using rotatory chair testing with electronystagmography (ENG) and the video Head Impulse Test (vHIT). RESULTS: Forty-five subjects (49 ears) were enrolled. The phoneme scores significantly improved from 35% (IQR 11-50) pre-implantation to 84% (IQR 76-90) one year post-implantation. No correlation was observed between the Fazekas score and the one-year post-implantation phoneme score (rsp=0.003, p = 0.986). Signal loss in at least one SCCs was detected in 97.7% of subjects and 77.8% of ears. There was no correlation between vestibular test results and fluid signal loss in the SCCs on MRI. CONCLUSION: Most individuals with DFNA9 show improved speech recognition with CI. The observed variability in CI outcomes was not linked to the Fazekas score. Additionally, our study confirms a high prevalence of focal sclerosis in DFNA9. Recognizing the limitations of this study, further research is needed to explore the predictive role of the Fazekas score on CI outcomes and its relationship with vestibular function.

Changes in participatory and societal outcomes during the waiting period for cochlear implantation - an observational study.

INTRODUCTION: Various factors, including an aging population and expanding eligibility criteria, may increase the demand for cochlear implants (CIs), potentially resulting in longer waiting times. In most Dutch CI centers, the time between referral and surgery exceeds 6 months. Clinical experience suggests that during the waiting period for cochlear implantation, hearing and communication difficulties increase. Simultaneously, there is an interest in outcomes more closely aligned with patient values and needs, which resulted in the SMILE (Societal Merit of Interventions on hearing Loss Evaluation) study. This paper presents results on observed changes in societal and participatory outcomes during waiting time in participants with a time to CI surgery exceeding 6 months. METHODS: SMILE is a prospective multi-center study including 232 individuals who were referred for unilateral CI. Continuous and nominal data from multiple questionnaires, sent immediately after referral and shortly before surgery, were analyzed by computing differences, Cohen's D, and odds ratios. RESULTS: Of the total 232 participants, 102 had a time between inclusion and surgery exceeding 6 months. Of these, 89 had (partially) filled out surveys at both time points. Of all the domain scores 55% did not show differences between timepoints. All Cohen's D estimates were relatively small, ranging from - 0.298 to 0.388 for those outcomes that showed noteworthy changes. CONCLUSION: Waiting time from referral to surgery, even though exceeding 6 months, was observed to not seriously affect non-clinically-prioritized patients in an adverse way. Future investigations should identify subgroups on tolerable waiting times regarding short- and long-term outcomes. TRIAL REGISTRATION: Trial registration number at ClinicalTrials.gov: NCT05525221, 25-8-2022.

Comparison of electrophysiological parameters between perimodiolar and lateral wall electrodes in paediatric cochlear implant users.

PURPOSE: (1) To compare the in vivo impedances of electrical contacts of the Nucleus Contour Advance (CA) perimodiolar electrodes and the Nucleus Slim Straight (SS) lateral wall electrodes. (2) To compare the relation between the electrode contact impedances and the behavioural T-/C-levels for both types of electrodes. METHODS: Retrospective case review in two quaternary otologic referral centres was performed. Data on the impedance of the electrode contacts and the T-/C-levels have been collected from 70 consecutive paediatric CI patients. RESULTS: (1) SS electrodes show significantly higher impedance values of the contacts compared to the CA electrodes. This can be explained by differences in the active surface of the electrode contacts and is true for the whole electrode arrays. (2) There are significant negative correlations observed between the impedances of the electrode contacts and the behaviourally measured T-/C-levels. (3) The strength correlation between the electrode impedances and the behavioural T-/C-levels is significantly higher and the T-/C-level variability is lower for the perimodiolar CA electrodes than for the lateral wall SS electrodes. CONCLUSIONS: There exist significant differences in the contact impedance between different cochlear implant electrode arrays. These differences could influence the implant programming parameters and should be considered in any protocols related to automatic implant programming based on objective measures. The predictive value of the electrode impedances for the behavioural T-/C-levels is higher for the perimodiolar CA electrodes than for the lateral wall SS electrodes.

Malleostapedotomy with the self-fixing and articulated titanium piston.

OBJECTIVE: To analyze the results of malleostapedotomy performed by applying the self-fixing and articulated titanium piston according to Häusler. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS AND INTERVENTIONS: This study concerns a retrospective analysis of the results of malleostapedotomy with the use of a self-fixing articulated titanium piston in 16 ears of 16 consecutively treated patients between 2005 and 2009. The medical files were used for the acquisition of data on medical and surgical history and to obtain pre- and postoperative audiometry. Diagnosis and outcomes of mainly revision surgeries are presented and compared to the literature. MAIN OUTCOME MEASURES: Effect of (revision) malleostapedotomy by evaluating postoperative audiometry and air-bone gap closure. RESULTS: The postoperative air-bone gap closure was ≤ 10 dB in 9/16 (56%) ears and within ≤ 20 dB in 13/16 (81%) ears. The mean postoperative air-bone gap was 14.3 dB HL (0.5-2.0 kHz) and 17.3 dB HL (0.5-4.0 kHz). Postoperatively, there was no increase in bone conduction thresholds larger than 3 dB (0.5-2.0 kHz) and postoperative dizziness was absent or very limited and transient. CONCLUSIONS: The malleostapedotomy procedure has become surgically less demanding over time by the technical improvements present in the nowadays available pistons. The design of the self-fixing and articulated titanium piston used in the present group of patients allows a safe and straight-forward malleostapedotomy procedure. Present hearing outcomes match with results presented in the literature.

Evaluation of an abutment-level superpower sound processor for bone-anchored hearing.

OBJECTIVES: Performance of an abutment-level superpower sound processor for bone-anchored hearing, the Ponto 3 SuperPower from Oticon Medical (BCD2), was compared to an earlier model from Oticon Medical (BCD1). DESIGN: A comparative study in which each patient serves as its own control. SETTING: Tertiary clinic. PARTICIPANTS: Eighteen experienced BCD1 users with profound mixed hearing loss. MAIN OUTCOME MEASURES: Speech reception thresholds in noise; APHAB and SSQ questionnaires. RESULTS: In a group of 18 patients with severe mixed hearing loss, the performance of a recently introduced bone conduction device (BCD2) is evaluated relative to that of an earlier model (BCD1). Speech reception thresholds for the sentence-in-noise test in the speech and noise frontal condition are not significantly different (P > .05) for BCD1 and BCD2. Speech reception thresholds for frontal speech and three identical noise sources are 1.7 dB lower for BCD2 than for BCD1 (P < .05). With the APHAB questionnaire, the score for background noise is significantly lower (P < .01), that is more favourable, for BCD2 than for BCD1 with an effect size of 0.91. The APHAB domain scores for ease-of-communication, reverberation and aversiveness of loud sounds are not significantly different for both devices (P > .05). Scores for the speech, spatial and quality of hearing domains of the SSQ questionnaire are significantly higher (P < .01), that is more favourable, for BCD2 than for BCD1 with effect sizes of 1.22, 0.71 and 1.05, respectively. Scores for the SSQ-factors "speech understanding," "spatial," "clarity, separation and identification" and "listening effort and concentration" were all significantly higher (P < .05) for BCD2 than for BCD1, with effect sizes of 1.28, 0.64, 0.98 and 0.78, respectively. On a proprietary questionnaire, 16 patients indicate a preference for BCD2 over BCD1 for conversations in a small group and two patients have no preference for either device. In a large group, one patient prefers BCD1, six patients have no preference, and eleven patients prefer BCD2. When listening to music, all patients prefer BCD2 over BCD1, with a strong preference for BCD2 for seven patients. When asked for an overall preference, all patients prefer BCD2 over BCD1, with nine patients strongly preferring BCD2. CONCLUSIONS: The lower speech reception thresholds in noise with BCD2 relative to BCD1 can be attributed to the "speech omni" directionality mode of BCD2. The combination of an improved directionality for primarily low-level inputs and a higher maximum force output, relevant for mid- and high-level inputs, results in a clear preference for BCD2 over BCD1.

On the evaluation of a superpower sound processor for bone-anchored hearing.

OBJECTIVES: Performance of a superpower bone-anchored hearing aid (Baha), the Baha Cordelle from Cochlear Bone-Anchored Solutions (BCD1), was compared to its successor, the Baha 5 SuperPower (BCD2). DESIGN: A comparative study in which each patient served as its own control. SETTING: Tertiary clinic. PARTICIPANTS: Ten experienced BCD1 users with profound mixed hearing loss. For comparison, data from another study with 10 experienced users with a severe mixed hearing loss using a Cochlear Baha 5 power sound processor (BCD-P) were included. MAIN OUTCOME MEASURES: Speech reception thresholds in noise and APHAB and SSQ questionnaires. RESULTS: Speech reception thresholds for the digits-in-noise (DIN) test were significantly lower (P < 5%), that is more favourable, for BCD2 in the speech and noise frontal condition and in the speech frontal and noise contralateral condition than for BCD1. For the group with severe mixed loss fitted with BCD-P, the SRTs were not significantly different (P > 5%) from the BCD2 values. With the APHAB questionnaire scores were significantly lower, that is more favourable, for the ease of communication (P < 5%) and the background noise (P < 1%) domains for BCD2 than for BCD1. APHAB scores for the aversiveness of loud sounds domain were not significantly different for both devices (P > 5%). Scores for the speech and quality domains of the SSQ questionnaire were significantly higher, that is more favourable, for BCD2 than for BCD1. APHAB and SSQ scores for BCD-P were not significantly different from those for BCD2 (P > 5%). CONCLUSIONS: Data for BCD2 in profound mixed loss are similar to those for BCD-P and a severe mixed loss. Of 10 patients, 2 expressed a strong preference for BCD2 over BCD1, and 7 patients had a preference for BCD2 over BCD1. One patient preferred BCD1 because of its built-in telecoil facility.

CT findings of the temporal bone in CHARGE syndrome: aspects of importance in cochlear implant surgery.

To provide an overview of anomalies of the temporal bone in CHARGE syndrome relevant to cochlear implantation (CI), anatomical structures of the temporal bone and the respective genotypes were analysed. In this retrospective study, 42 CTs of the temporal bone of 42 patients with CHARGE syndrome were reviewed in consensus by two head-and-neck radiologists and two otological surgeons. Anatomical structures of the temporal bone were evaluated and correlated with genetic data. Abnormalities that might affect CI surgery were seen, such as a vascular structure, a petrosquamosal sinus (13 %), an underdeveloped mastoid (8 %) and an aberrant course of the facial nerve crossing the round window (9 %) and/or the promontory (18 %). The appearance of the inner ear varied widely: in 77 % of patients all semicircular canals were absent and the cochlea varied from normal to hypoplastic. A stenotic cochlear aperture was observed in 37 %. The middle ear was often affected with a stenotic round (14 %) or oval window (71 %). More anomalies were observed in patients with truncating mutations than with non-truncating mutations. Temporal bone findings in CHARGE syndrome vary widely. Vascular variants, aberrant route of the facial nerve, an underdeveloped mastoid, aplasia of the semicircular canals, and stenotic round window may complicate cochlear implantation.

Patients with Pendred syndrome: is cochlear implantation beneficial?

OBJECTIVE: To evaluate the benefit of cochlear implantation in patients with Pendred syndrome. DESIGN: Retrospective study. SETTING: Tertiary centre. PARTICIPANTS AND MAIN OUTCOME MEASURES: Speech perception was measured using a phonetically balanced word list at a sound pressure level of 65 dB. Post-operative phoneme scores at 12-month for adults and 36-month for children with Pendred syndrome were compared to scores of patients with an enlarged vestibular aqueduct (EVA) and a reference group with an unknown cause of hearing impairment. Quality of life was measured with the Nijmegen Cochlear Implant Questionnaire to evaluate the differences between pre- and post-implantation. RESULTS: The mean post-operative phoneme scores were as follows: in the Pendred group, 91% (n = 16; SD = 10) for children and 78% (n = 7; SD = 14) for adults; in the reference group, 79% (n = 59; SD = 20) for children and 73% (n = 193; SD = 18) for adults; and in the EVA group, 84% (n = 6; SD = 7) for children and 66% (n = 12; SD = 22) for adults. A significant difference in speech perception was found between the children of the Pendred group and the reference group of 11.4% (SE = 5.2; P = 0.031). Between the adults, a difference of 11.2% (SE = 6.7; P = 0.094) was found. The difference between the Pendred group and the EVA group was 5.7%(SE = 4.5; P = 0.22) for children and 9.9% (SE = 8.7; P = 0.28) for adults. A significant improvement post-implantation in four of the six subdomains of the quality of life questionnaire was found: basic sound perception (P = 0.002), advanced sound perception (P = 0.004), speech production (P = 0.018) and activity limitations (P = 0.018). The two not significant subdomains were self-esteem (P = 0.164) and social interaction (P = 0.107). CONCLUSIONS: After cochlear implantation, children with Pendred syndrome performed better than the reference group with respect to speech perception, however, adults performed similar. No significant differences were found between the Pendred and EVA group. Consequently, during pre-operative counselling, the two groups of patients may be considered comparable in terms of expected speech perception performance after cochlear implantation.

Stable long-term outcomes after cochlear implantation in subjects with TMPRSS3 associated hearing loss: a retrospective multicentre study.

BACKGROUND: The spiral ganglion hypothesis suggests that pathogenic variants in genes preferentially expressed in the spiral ganglion nerves (SGN), may lead to poor cochlear implant (CI) performance. It was long thought that TMPRSS3 was particularly expressed in the SGNs. However, this is not in line with recent reviews evaluating CI performance in subjects with TMPRSS3-associated sensorineural hearing loss (SNHL) reporting overall beneficial outcomes. These outcomes are, however, based on variable follow-up times of, in general, 1 year or less. Therefore, we aimed to 1. evaluate long-term outcomes after CI implantation of speech recognition in quiet in subjects with TMPRSS3-associated SNHL, and 2. test the spiral ganglion hypothesis using the TMPRSS3-group. METHODS: This retrospective, multicentre study evaluated long-term CI performance in a Dutch population with TMPRSS3-associated SNHL. The phoneme scores at 70 dB with CI in the TMPRSS3-group were compared to a control group of fully genotyped cochlear implant users with post-lingual SNHL without genes affecting the SGN, or severe anatomical inner ear malformations. CI-recipients with a phoneme score ≤ 70% at least 1-year post-implantation were considered poor performers and were evaluated in more detail. RESULTS: The TMPRSS3 group consisted of 29 subjects (N = 33 ears), and the control group of 62 subjects (N = 67 ears). For the TMPRSS3-group, we found an average phoneme score of 89% after 5 years, which remained stable up to 10 years post-implantation. At both 5 and 10-year follow-up, no difference was found in speech recognition in quiet between both groups (p = 0.830 and p = 0.987, respectively). Despite these overall adequate CI outcomes, six CI recipients had a phoneme score of ≤ 70% and were considered poor performers. The latter was observed in subjects with residual hearing post-implantation or older age at implantation. CONCLUSION: Subjects with TMPRSS3-associated SNHL have adequate and stable long-term outcomes after cochlear implantation, equal to the performance of genotyped patient with affected genes not expressed in the SGN. These findings are not in line with the spiral ganglion hypothesis. However, more recent studies showed that TMPRSS3 is mainly expressed in the hair cells with only limited SGN expression. Therefore, we cannot confirm nor refute the spiral ganglion hypothesis.